Bullous pemphigoid: a correlative study of autoantibodies, circulating immune complexes and dermo-epidermal deposits.
Twenty bullous pemphigoid (BP) patients were studied to establish any correlation between free anti-basement membrane zone (BMZ) antibodies, circulating immune complexes (CIC) and dermo-epidermal junction deposits. CIC levels were evaluated by 2% polyethylene glycol (PEG) precipitation. The twenty patients were found to have IgG and/or C3 deposited in the BMZ. Eight of the twelve patients who had no free anti-BMZ antibodies displayed a positive in vivo C4 and/or CIq staining and high levels of CIC. Moreover, CIC were detected in only one patient with positive circulating free anti-BMZ antibodies. The presence of free anti-BMZ antibodies was generally found to correlate with the absence of cutaneous deposits of CIq and /or C4 and with negative CIC; on the other hand, the absence of free anti-BMZ antibodies was generally found to correlate with high levels of CIC and with deposits of C3 and CIq and/or C4. The absence of circulating free anti-BMZ antibodies in BP patients, could be explained by the formation of CIC. It is possible that BMZ antigens released from damaged tissue could combine with free antibodies and form complexes in the blood. The release could involve locally formed immune complexes. Elevated CIC levels were generally found to correlate with the presence of active disease.
D000368:Aged; D000936:Antigen-Antibody Complex / Q000032:analysis*; D001323:Autoantibodies / Q000032:analysis*; D001485:Basement Membrane / Q000276:immunology; D003166:Complement Activating Enzymes / Q000032:analysis; D015922:Complement C1q; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D005779:Immunodiffusion; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000276:immunology*; D012867:Skin / Q000276:immunology*; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology*
D000936:Antigen-Antibody Complex; D001323:Autoantibodies; D003176:Complement C3; D003181:Complement C4; D007074:Immunoglobulin G; D015922:Complement C1q; D003166:Complement Activating Enzymes